Superior vena cava obstruction - Case Study

A 65 years old man  who is a smoker with no h/o hypertension or diabetes presented with frequent episodes of difficult breathing and cough for last 6 months. Cough is present throughout the day and night and is is usually dry, sometimes associated with scanty mucoid expectoration. He also noticed small amount of blood with sputum several times. The patient also complains of loss of appetite and substantial weight loss for the same duration. There is no history of fever, chest pain or contact with TB patients.
For the last few days, he has noticed gradual swelling of the face and arms along with shortness of breath, which is more marked on exertion. The patient also complains of headache which is
aggravated by cough and movement of the head for the same duration. He denies any change of
voice, back pain, yellow coloration of urine and sclera. His bowel and bladder habits are normal.
He smokes about 30 sticks/day for 25 years.

On Examination: the chest movement during respiration were decreased on the right side, and the percussion note was dull in the right upper chest. On auscultation of the lung fields, there were decreased breath sounds on the right upper chest, and few crepitations were heard throughout.
Heart sounds were normal.
Fundoscopy shows dilated vessels, hemorrhage and exudates.
Other systemic examination was normal.

Provisional Diagnosis: Superior vena cava (SVC) obstruction

Q. What do you think the cause of SVC obstruction in this case?
According to the age of the patient in this case 65 years old so causes are:

• bronchial carcinoma, 
• lymphoma

In young or early age—common cause is lymphoma.

Q. Tell one single investigation which will help the diagnosis of SVC obstruction.
Chest X-ray (which may show bronchial carcinoma and lymphoma).

Q. What investigations should be done in SVC obstruction?
 As follows:
1. Chest X-ray
2. CBC, ESR
3. Sputum for malignant cells
4. CT or MRI of chest
5. Others (according to suspicion of cause or physical findings):

•  If palpable lymph nodes FNAC or biopsy
•  Bronchoscopy and mediastinoscopy, venography and occasionally thoracotomy may be needed
•  Echocardiography in some cases.

Q. What physical findings did you look for to find out the cause of SVC obstruction?

 As follows:

•  Chest (bronchial carcinoma)
•  Lymph nodes (lymphoma and metastasis)
•  Liver and spleen (lymphoma)
•  Clubbing, nicotine stain (bronchial carcinoma)
•  Thyroid (to see retrosternal thyroid).

Q. What are the presentations of SVC obstruction?

The patient may complain of:

•  Breathlessness, cough, hoarseness of voice and dysphagia
•  Flushing, red, puffy and edematous face
•  Headache (early morning), which is severe with coughing. 
•  May be syncope, dizziness or blackout,stupor, seizure (due to increased intracranial pressure)
• Symptoms are aggravated on lying down or bending forward (indicates mediastinal involvement)
•  The patient may have stridor (tracheal compression), hoarseness of voice (recurrent laryngeal nerve involvement), Horner’s syndrome (cervical sympathetic chain involvement).

Features of mediastinal compression—according to the involvement of structure:

1. Trachea: breathlessness, cough, stridor.

2. Esophagus: Dysphagia.

3. Bronchus: Features of collapse of lung.

4. Recurrent laryngeal nerve: Hoarseness of voice, bovine cough.

5. Phrenic nerve: Breathlessness (X-ray chest shows raised diaphragm).

6. Cervical sympathetic chain involvement: Horner’s syndrome.

7. SVC:

xx Face is puffy, edematous and red

xx Neck is swollen with engorged, non-pulsatile neck veins

xx Visible tortuous veins in chest wall and abdomen, flow is downwards

xx Upper limbs are edematous with prominent engorged veins.

Q. What are the causes of death in SVC obstruction?

 Death is due to:

•  Respiratory obstruction
•  Intracranial hemorrhage

Q. What are the causes of SVC obstruction?

 As follows:

1. Bronchial carcinoma (common cause, in 75%)

2. Lymphoma (early age, also in elderly)

3. Other causes:

•  Retrosternal thyroid
•  Thymoma
• Mediastinal fibrosis (its causes include idiopathic, radiation, methysergide used in migraine and histoplasmosis)
•  Metastasis to the mediastinum
•  Chronic constrictive pericarditis
•  Giant aortic aneurysm
•  Carcinoma of esophagus
•  Rarely thrombosis, invasion by malignancy, germ cell tumor.

Q. What is Pemberton’s test?

 On lifting the arms over the head for more then 1 minute, there is increased facial plethora or

cyanosis, raised JVP (non-pulsatile) and inspiratory stridor.

Q. How will you treat the case?

Treatment is according to cause:

1. General treatment:

•  To relieve edema—intravenous frusemide, head should be raised, dexamethasone may be used
•  To relieve severe obstruction—expandable metallic stent may be used (placed in SVC) as palliative measure. Venoplasty may be considered
•  Anticoagulant may be given, if needed
•  Ventilatory support, if needed.

2. Treatment of cause:

•  The most common cause is bronchial carcinoma. Radiotherapy in non-small cell carcinoma and chemotherapy for small cell carcinoma.
• If lymphoma—treat accordingly (usually chemotherapy).